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Once a month, Frieden plans to meet with a CDC employee to discuss his or her public health work. “I get to learn more about the great work CDC scientists do,” said Frieden, in launching this new series earlier this year.

Staff are nominated by senior leadership to participate in Conversations with the Director. If you are interested, contact your senior leaders to submit your name or the name of someone else you think would be good for this series.

What Is Sickle Cell Disease?

Sickle cell disease (SCD) is a group of inherited red blood cell disorders. In SCD, the red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle.” The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such as infection, acute chest syndrome, and stroke.

SCD is a genetic condition that is present at birth. It is inherited when a child receives two sickle cell genes—one from each parent.

SCD is diagnosed with a simple blood test. It most often is found at birth during routine newborn screening tests at the hospital. In addition, SCD can be diagnosed before birth. Because children with SCD are at an increased risk of infection and other health problems, early diagnosis and treatment are important.

People with SCD start to have signs of the disease during the first year of life, usually around 5 months of age. Symptoms and complications of SCD are different for each person and can range from mild to severe. There is no single best treatment for all people with SCD. Treatment options are different for each person, depending on the symptoms.

The two discussed sickle cell disease. SCD is a group of inherited red blood cell disorders. Photo by Kathy Nellis

Impact and CDC’s Role

Sickle cell disease (SCD) is a common inherited blood disorder in the United States, affecting an estimated 90,000 to 100,000 Americans. SCD can lead to lifelong disabilities and reduce average life expectancy. In addition, the financial cost of SCD is high, both to people with the disease and to the healthcare system. CDC’s National Center on Birth Defects and Developmental Disabilities, Division of Blood Disorders, considers SCD a major public health concern and is committed to conducting surveillance, raising awareness, and promoting health education.

Currently, there are no data systems in the United States to determine the number of people who have SCD and other hemoglobinopathies (disorders affecting red blood cells), nor to fully describe how these conditions affect an individual’s health. CDC is working with the National Heart, Lung, and Blood Institute (NHLBI) to implement a pilot data collection system for hemoglobinopathies called RuSH—the Registry and Surveillance System for Hemoglobinopathies.

“My pride and joy is the RuSH program,” Grant told Frieden. “When I joined the Division of Blood Disorders we did not have any work in sickle cell. I had a real desire to help create a program around sickle cell like this.”

"This is just the second one of these conversations I have had,” said Frieden, and “they are a real treat for me because we have so many great people at CDC. Hearing your expertise in surveillance, you are faced with a hard problem and you say, ‘Well, this is fun.’ It’s just a great attitude and it’s what makes CDC a great institution… “And your comment, ‘I need to be able to explain to my grandmother the importance of what I am doing’—I think that is very much the heart of what we can and should do here.”Photo by Kathy Nellis

Grant is the project director for the RuSH program, the first population-based public health surveillance system for sickle cell disease and thalassemia, conducted in collaboration with NIH/NHLBI. “RuSH is using innovative surveillance methods to not only determine how many people have sickle cell disease or thalassemia, but also identify those communities that are in need of greater medical resources to address SCD and thalassemia., monitor how SCD and thalassemia affect people across their lifespan, and identify the medical and social needs of people with SCD and thalassemia across their lifespan,” she explains. In addition, Grant and her team are developing health education materials to increase knowledge and awareness about sickle cell disease and sickle cell trait among the general public, as well as among the healthcare and public health community in the US and globally.

Grant and CDC

Grant, who received her PhD in biochemistry and molecular biology from Emory University, joined CDC in 2002 as an Epidemic Intelligence Service officer assigned to the Division of Reproductive Health. She worked there until 2007 when she became Epidemiology and Surveillance team lead in the Division of Blood Disorders. In 2010 she became chief of the Epidemiology and Surveillance Branch in DBD. Grant, who is also a commander in the US Public Health Service, serves as a recognized national leader championing public health programs for sickle cell disease.

Grant discussing the global and US burden of sickle cell disease with Oscar-winning actor and UNESCO Goodwill Ambassador Forest Whitaker at the home of US Ambassador David Killian in Paris France in June 2011. They were there to attend a special dinner in honor of Antoinette Sassou-Nguesso, First Lady of Congo-Brazzaville and activist for health issues and peace in Africa. Grant was in Paris serving as a US representative on behalf of the State Department for the 3rd UN World Sickle Cell Days.

In 2009 she received a commendation medal from the US Public Health Service in recognition for developing programs to improve the health of individuals affected by sickle cell disease. Grant wrote the proposal for the RuSH program while she was on maternity leave with her fourth child. “It was 200 pages long,” she recalls. Her diligence paid off as the program received $11 million dollars to fund seven states to do surveillance work in sickle cell. “The states had two years for the project and right now are in the process of finishing and sending us the data.”

Idea Exchange

The hour-long conversation served as a valuable exchange of information. The two discussed the challenges in surveillance and also the challenges in tackling the disease. “There’s not much understanding of the burden of sickle cell,” noted Frieden.

Right now there are not accurate mortality statistics and even getting health data is tricky, Grant said. “We’ve only been doing newborn screenings since 2006, and that misses many adults and the large number of immigrants who move here with sickle cell.”

She continued, “But the RuSH program has been great. I think we are going to be putting out a lot of information that will be shocking to people. Just the enormous pieces of data we have gotten so far have blown our mind and I think we are going to find more people than we thought were out there, a lot more mortality, a lot more extreme hospitalizations.”

Frieden asked Grant, “Is it possible to begin a dialogue about prevention?” “Absolutely,” she answered. “The timing is good right now because there is so much focus in the media on trait.”

Frieden pointed out that “trait seems to increase the risk of sudden death with extreme exercise,” noting the interest in testing for sickle cell in the National Collegiate Athletic Association and the military. He and Grant discussed how focusing on universal precautions can be more effective than testing. “The benefit of increased media attention is that everyone is talking about trait right now,” said Grant, “and when we educate about exercise, we can also educate about reproductive choices.”

Collegial Conversation

There was friendly personal conversation too, as Frieden asked Grant about her background. She grew up in New Jersey and went to Rutgers for her undergraduate work. “I was the first person in my family to finish college. My parents were postal workers; my grandfather was a cook, and my grandmother a maid. They were all very focused on my getting an education. I couldn’t watch TV!”

The two discussed her education, her career and heritage, and Frieden’s hope to provide more opportunities for others to follow in her footsteps. “We need to do better at CDC about having racial and ethnic minorities represented in leadership positions. I am committed to that.”

Grant decided to switch careers during a difficult period in her life. “At the time I had a lot of struggles, four people in my family were dying of HIV, I had some health issues, and I just wanted my life to mean more, in a practical way. Public health seemed like that was where I needed to be to address those issues. Becoming a geneticist did not seem practical; I could not explain to my grandma what I did, so I decided to go into public health. And I feel so wonderful about that decision.”

Grant with CDC colleague Azfar Siddiqi (right) and Fadhil Ibtihal (World Health Organization, EMRO) in Jordan in June 2011 discussing strategy at the Intercountry Meeting to Develop Regional Guidelines for the Development and Implementation of National Strategies and Programs for the Prevention and Management of Hemoglobinopathies and Genetic Diseases in Eastern Mediterranean Region.Larger image

Frieden and Grant talked about epi info and public health tools, their families, the joys and challenges of children (she has four, he has two sons), and even books.

“Did you read the recent biography of Henrietta Lacks?” he asked.

“Oh, it was one of my favorites, ” Grant said, “I cried and cried.”

“It was very moving,” agreed Frieden.

“It made me look at things so differently,” said Grant. “It reminded me how important it is how people perceive things, particularly as a scientist. When you work on human cells, you take so much for granted. This was a real person with a real family. That was amazing.”

“It was an amazing book,” said Frieden.

The hour passed by quickly for both.

“This is just the second one of these conversations I have had,” Frieden told Grant, “and they are a real treat for me because we have so many great people at CDC. Hearing your expertise in surveillance, you are faced with a hard problem and you say, ‘Well, this is fun.’ It’s just a great attitude, and it’s what makes CDC a great institution…

“And your comment, ‘I need to be able to explain to my grandmother the importance of what I am doing’ —I think that is very much the heart of what we can and should do here.”